Adrenal Disease Flashcards

1
Q

What is Cushing’s syndrome?

A

Symptoms of increased circulating glucocorticoid

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2
Q

What are the common causes of Cushing’s?

A
Exogenous administration of steroids
Increased ACTH
-pituitary (65%)
-ectopic (15%)
Excess adrenal cortisol
-adrenal tumour/nodular hyperplasia (25%)
-subsequent ACTH suppression
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3
Q

What are the symptoms of Cushing’s?

A
Central weight gain
Change of appearance
Depression
Insomnia
Poor libido
Thin skin/easy bruising
Excess hair growth/acne
Sx of DM
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4
Q

What are the signs of Cushing’s?

A
Moon face
Frontal balding
Striae
Hypertension
Pathological fractures
Dorsal fat pad (buffalo hump)
Proximal myopathy
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5
Q

How is Cushing’s diagnosed?

A

Raised cortisol
Overnight dexamethasone suppression test
Plasma ACTH

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6
Q

Describe the overnight dexamethasone suppression test

A

1mg oral dexamethasone given at midnight
Serum cortisol checked before and at 8am
-normal neg feedback leads to cortisol <50mmol/L
-Cushing’s - failure to suppress

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7
Q

What is Addison’s disease?

A

A primary adrenal insufficiency resulting from destruction of the entire adrenal cortex

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8
Q

What hormone deficiencies are present in Addison’s disease?

A

Glucocorticoid (cortisol)
Mineralocortiocoid
Sex-steroids

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9
Q

What are the causes/risk factors for Addison’s disease?

A
Autoimmune (80% UK)
TB (most common worldwide)
Overwhelming sepsis
Metastatic lung/breast cancer
Lymphoma
Adrenal haemmorhage
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10
Q

What are the symptoms of Addison’s disease?

A
Weight loss
Malaise
Weakness
Myalgia
Syncope
Depression
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11
Q

What are the signs of Addison’s disease?

A

Pigmentation (new scars, palmar creases)
Postural hypotension
Signs of dehydration
Loss of body hair

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12
Q

How is Addison’s diagnosed?

A

FBC (anaemia)
U&Es (low sodium, high potassium, uraemia)
Raised Ca
Low glucose
Synacthen test (raised cortisol excludes Addison’s)
9am Cortisol (raised ACTH & low/normal cortisol =dx)
CXR/adrenal CT

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13
Q

Describe the Synacthen test

A

Give ACTH analogue
Measure plasma cortisol before and 30 minutes after
>550nmol/L excludes Addison’s

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14
Q

Describe the 9am ACTH test

A

Raised ACTH w/ low/normal cortisol = Addison’s

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15
Q

What is an Addisonian crisis?

A
Severely inadequate levels of cortisol
Presents w/
-fever
-nausea/vomiting
-shock
-hypoglycaemia
-hypernatremia &amp; hypokalaemia
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16
Q

What is Conn’s syndrome?

A

Adrenal adenoma leading to primary hyperaldosteronism

Responsible for 60% of primary hyperaldosteronism

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17
Q

What are the main physiological effects of hyperaldosteronism?

A

Sodium and water retention

Knock on hypokalaemia

18
Q

What are the symptoms of Conn’s syndrome?

A

Mostly asymptomatic

19
Q

What are the signs of Conn’s syndrome?

A
Hypertension (treatment resistant, causing headaches)
Features of hypokalaemia
-cramps
-weakness
-tetany
-polyuria
20
Q

What are the biochemical signs of Conn’s syndrome?

A

Hypokalaemia w/ urinary K loss

Elevated plasma aldosterone:renin

21
Q

How is Conn’s syndrome distinguished from adrenal hyperplasia?

A
Adrenal CT
Adrenal scintography (unilateral uptake of isotope)
22
Q

What is a Phaechromocytoma?

A

Catecholamine secreteing tumours arising from sympathetic paraganglial cells (Chromaffin cells)

23
Q

Where are Phaechromocytomas commonly found?

A

> 70% Adrenal medulla
10% extra-adrenal
10% are bilateral
10% familial (MEN IIa/IIb, NF, VHL syndrome)

24
Q

How do Phaechromocytomas present?

A

Severe/episodic HTN unresponsive to treatment

Vague/episodic symptoms in several systems

25
Q

What are the general symptoms of Phaechromocytomas?

A

Vague and episodic

Sweating, heat intolerance, pallor/flushing

26
Q

What are the neurological symptoms of Phaechromocytomas?

A

Neurological - headaches, visual disturbances, seizures

27
Q

What are the CV symptoms of Phaechromocytomas?

A

CV - palpitations, chest tightness, dyspnoea, postural hypertension

28
Q

What are the GI symptoms of Phaechromocytomas?

A

GI - abdominal pain, nausea, constipation

29
Q

How are Phaechromocytomas diagnosed?

A
Urine collections (raised metadrenaline/normetadrenaline)
Imaging to locate the tumour
30
Q

What is the appropriate management for Phaechromocytomas?

A

Alpha blockade (phenoxybenzamine)
B-blockers (after a-blockade)
Surgical excision

31
Q

What is the management for Addison’s disease?

A

12-25mg Hydrocortisone daily (t.d.s.)
50-200mg Fludrocortisone daily
Increase doses for exercise
Double doses for surgery/febrile illness/trauma

32
Q

What is the main side effect of Hydrocortisone?

A

Insomnia

Avoid giving late in day

33
Q

What are the indications for giving Fludrocortisone in Addison’s disease?

A

Postural hypotension
Low Na
High K

34
Q

What is Congenital Adrenal Hyperplasia (CAH)?

A

Congenital deficiency in 21-a-hydroxylase
Cannot produce mineralocorticoids/glucocorticoids
Can produce sex hormones

35
Q

What are the main hormonal changes in CAH?

A

Aldosterone/cortisol decreased

Testosterone raised

36
Q

How does CAH present in females?

A

Virilisation of external genitalia (clitoral hypertrophy, labial fusion)

37
Q

How does CAH present in males?

A

Enlarged penis/pigmented scrotum
Salt-losing crisis (80%) at 1-3wks of age
Hypervirilisation (early pubarche, adult BO, muscular build)

38
Q

What investigations are appropriate in CAH?

A

17-a-hydroxyprogesterone (raised, =dx)
Low Na, high K
Metabolic acidosis

39
Q

What is the management for CAH?

A

Counselling
Steroid cover, as per Addison’s
At risk of Addisonian crises

40
Q

What is the management for Conn’s syndrome?

A

Laporoscopic adrenalectomy

Spironolactone (treatment of choice for bilateral hyperplasia)

41
Q

What is the 2nd most common cause of primary hyperaldosteronism?

A

Bilateral adrenal hyperplasia 30%